Angiodysplasia

血管发育不良
  • 文章类型: Case Reports
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  • 文章类型: Letter
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  • 文章类型: Randomized Controlled Trial
    背景:小肠复发性出血占消化道出血病例的5%至10%,仍然是治疗挑战。沙利度胺已被评估用于治疗由于小肠血管发育不良(SIA)引起的复发性出血,但缺乏确证试验。
    方法:我们进行了多中心,双盲,随机化,安慰剂对照试验研究沙利度胺治疗SIA所致复发性出血的疗效和安全性.因SIA导致的复发性出血(前一年至少有四次出血)的符合条件的患者被随机分配接受口服每日剂量为100mg或50mg的沙利度胺或安慰剂,为期4个月。在4个月治疗期结束后,对患者进行至少1年的随访。主要终点是有效反应,定义为与治疗前一年相比,沙利度胺治疗结束后一年内发生的出血事件数量减少至少50%。关键次要终点是停止出血而不再出血。输血,因为出血住院,出血持续时间,和血红蛋白水平。
    结果:总体而言,150例患者接受随机分组:51例进入100-mg沙利度胺组,50mg沙利度胺组49,安慰剂组50。100mg沙利度胺组有效反应的患者百分比,50毫克沙利度胺组,安慰剂组为68.6%,51.0%,和16.0%,分别(三组同时比较P<0.001)。次要终点的分析结果支持主要终点的分析结果。与安慰剂组相比,沙利度胺组的不良事件更常见;具体事件包括便秘,嗜睡,肢体麻木,外周水肿,头晕,和肝脏酶水平升高。
    结论:在这项安慰剂对照试验中,沙利度胺治疗可减少因SIA引起的复发性出血患者的出血.(由国家自然科学基金委员会和上海市教委资助,高峰临床医学;临床试验。gov编号,NCT02707484。).
    BACKGROUND: Recurrent bleeding from the small intestine accounts for 5 to 10% of cases of gastrointestinal bleeding and remains a therapeutic challenge. Thalidomide has been evaluated for the treatment of recurrent bleeding due to small-intestinal angiodysplasia (SIA), but confirmatory trials are lacking.
    METHODS: We conducted a multicenter, double-blind, randomized, placebo-controlled trial to investigate the efficacy and safety of thalidomide for the treatment of recurrent bleeding due to SIA. Eligible patients with recurrent bleeding (at least four episodes of bleeding during the previous year) due to SIA were randomly assigned to receive thalidomide at an oral daily dose of 100 mg or 50 mg or placebo for 4 months. Patients were followed for at least 1 year after the end of the 4-month treatment period. The primary end point was effective response, which was defined as a reduction of at least 50% in the number of bleeding episodes that occurred during the year after the end of thalidomide treatment as compared with the number that occurred during the year before treatment. Key secondary end points were cessation of bleeding without rebleeding, blood transfusion, hospitalization because of bleeding, duration of bleeding, and hemoglobin levels.
    RESULTS: Overall, 150 patients underwent randomization: 51 to the 100-mg thalidomide group, 49 to the 50-mg thalidomide group, and 50 to the placebo group. The percentages of patients with an effective response in the 100-mg thalidomide group, 50-mg thalidomide group, and placebo group were 68.6%, 51.0%, and 16.0%, respectively (P<0.001 for simultaneous comparison across the three groups). The results of the analyses of the secondary end points supported those of the primary end point. Adverse events were more common in the thalidomide groups than in the placebo group overall; specific events included constipation, somnolence, limb numbness, peripheral edema, dizziness, and elevated liver-enzyme levels.
    CONCLUSIONS: In this placebo-controlled trial, treatment with thalidomide resulted in a reduction in bleeding in patients with recurrent bleeding due to SIA. (Funded by the National Natural Science Foundation of China and the Shanghai Municipal Education Commission, Gaofeng Clinical Medicine; ClinicalTrials.gov number, NCT02707484.).
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  • 文章类型: Journal Article
    目标:评估患病率,死亡率,和1990年至2019年血管肠道疾病(VID)的残疾调整寿命年(DALYs)。方法:本研究利用2019年全球疾病负担研究进行了二次数据分析。患病率,按性别分析VID的死亡率和DALY,年龄和社会人口指数(SDI),分别。使用R软件进行分析。结果:全球,流行的VID病例数量从2019年的1,990-175,740(157,941-198,969)的100,158(95%不确定区间:89,428-114,013)增加。然而,在1990年至2019年期间,VID患病率的年龄标准化率(ASR)从每100,000人口2.47(95%不确定区间:2.24~2.76)下降至每100,000人口2.21(1.98~2.48).此外,从1990年到2019年,死亡率的ASR也有所下降。在1990年至2019年期间,中高水平地区的疾病负担最高。结论:全球,从1990年到2019年,与VID相关的疾病负担有所下降。然而,在被分类为高和中高SDI的国家中,仍需要采取协调一致的努力来加强打击VID的措施。
    Objectives: Assess the prevalence, mortality, and disability-adjusted life years (DALYs) of vascular intestinal disorders (VID) from 1990 to 2019. Methods: This study conducted a secondary data analysis utilizing the Global Burden of Diseases Study 2019. The prevalence, mortality and DALYs of VID were analyzed by sex, age and socio-demographic index (SDI), respectively. Analyses were performed by using R software. Results: Globally, the number of prevalent VID cases increased from 100,158 (95% uncertainty interval: 89,428-114,013) in 1,990-175,740 (157,941-198,969) in 2019. However, the age-standardized rates (ASR) of VID prevalence declined from 2.47 (95% uncertainty interval: 2.24-2.76) per 100,000 population to 2.21 (1.98-2.48) per 100,000 population between 1990 and 2019. Furthermore, the ASR of mortality also decreased from 1990 to 2019. Between 1990 and 2019, the regions with high and high-middle level exhibited the highest diseases burden. Conclusion: Globally, the diseases burden associated with VID demonstrated a decline from 1990 to 2019. However, concerted efforts are still required to enhance measures to combat VID within countries categorized as high and high-middle SDI.
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  • 文章类型: Case Reports
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  • 文章类型: Journal Article
    对接受经导管主动脉瓣植入术(TAVI)治疗的Heyde综合征(HS)患者的国际病例报告进行了系统评价,以探讨该组患者的临床特征和手术成功率。方法:电子数据库,包括PubMed,Embase和CNKI,使用搜索词的组合进行搜索,海德综合征,消化道出血,主动脉狭窄,血管发育不良和经导管主动脉瓣置换术。所有病例报告均根据纳入标准进行筛选,和HS患者数据进行总结。
    共有31例报告有主动脉瓣狭窄和反复消化道出血病史的患者。超声心动图(UCG)记录27例,包括那些严重的主动脉瓣狭窄(n=26)。据报道,胃肠道后遗症22例,十二指肠和空肠最常见(n=9)。在17例病例中测量了vonWillebrand因子(vWF-HMWM)的高分子量多聚体,其中大多数较低(n=15),少数正常(n=2)。所有患者在药物治疗和内镜治疗后出现复发性出血,经TAVI治疗后症状改善(31/31)。在TAVI后的11/12例中,vWF处于正常水平。对25例患者进行了随访,其中22例没有症状复发,HS患者的TAVI有效率为88%。
    HS的特征是血管发育不良,主动脉瓣狭窄和血管性血友病在药物和内镜治疗后频繁复发出血。TAVI是一种有效的治疗方法,分辨率为88%。
    UNASSIGNED: A systematic review of international case reports of patients with Heyde syndrome (HS) treated by transcatheter aortic valve implantation (TAVI) was conducted to explore the clinical characteristics of this group of patients and sirgical success. Methods: Electronic databases, including PubMed, Embase and CNKI, were searched with combinations of the search terms, Heyde syndrome, gastrointestinal bleeding, aortic stenosis, angiodysplasia and transcatheter aortic valve replacement. All case reports were screened according to inclusion criteria, and HS patient data was summarized.
    UNASSIGNED: A total of 31 case reports concerned patients with a history of aortic stenosis and repeated gastrointestinal bleeding. Ultrasonic cardiograms (UCG) were recorded for 27 cases, including those with critical aortic stenosis (n = 26). Gastrointestinal sequelae were reported in 22 cases with duodenal and jejunal being the most common (n = 9). High-molecular-weight multimers of von Willebrand Factor (vWF-HMWM) were measured in 17 cases with the majority being lower (n = 15) and the minority normal (n = 2). All patients experienced recurrent bleeding after medication and endoscopic therapy and symptoms improved after TAVI (31/31). vWF was at normal levels in 11/12 cases post-TAVI. Twenty-five patients were followed up and 22 had no recurrence of symptoms giving an efficacy rate of 88% for TAVI in HS patients.
    UNASSIGNED: HS is characterized by angiodysplasia, aortic stenosis and von Willebrand disease with frequent recurrence of bleeding after drug and endoscopic treatment. TAVI is an effective therapy with an 88% resolution rate.
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  • 文章类型: Case Reports
    背景:Heyde综合征在临床实践中很容易被忽视或误判,因为它具有多种疾病的共同临床表现,以及用于诊断Heyde三联症的几种相应检查的准确性有限。此外,由于抗凝和止血之间的矛盾,这些患者的主动脉瓣置换术通常会延迟。在这里,我们介绍了一个罕见的非典型Heyde综合征病例。患者的严重间歇性消化道出血即使通过局部肠管切除术也未完全治愈。在没有获得性血管性血友病综合征(AVWS)或血管发育不良的直接证据的情况下,她长期的消化道出血在接受经导管主动脉瓣植入术(TAVI)后终于停止.
    方法:一名64岁女性患有难治性消化道出血和劳累性呼吸困难。由于持续出血和反复输血,进行了局部肠切除术;随后,组织学检查显示血管发育不良。直到3年后才怀疑Heyde综合征,此时患者再次开始出血,经超声心动图检查发现主动脉瓣严重狭窄.因此,即使有出血倾向,当患者病情相对稳定时也进行了TAVI,但当时血管造影期间没有血管发育不良和AVWS的证据.患者上述症状经TAVI治疗后明显缓解,随访2年,无明显缺血或出血事件发生。
    结论:HMWM-vWFs的可见特征对于Heyde综合征的临床诊断不应必不可少。子宫内膜切除术可能是严重出血患者主动脉瓣置换术的桥接治疗,和TAVI可能对中度至高度手术风险患者有益,即使他们有潜在的出血风险.
    BACKGROUND: Heyde\'s syndrome can be easily overlooked or misjudged in clinical practice because it shares common clinical manifestations with multiple diseases as well as limited accuracy of several corresponding examinations for diagnosing Heyde\'s triad. Moreover, aortic valve replacement is often delayed in these patients due to the contradiction between anticoagulation and hemostasis. Herein, we present a rare case of atypical Heyde\'s syndrome. The patient\'s severe intermittent gastrointestinal bleeding was not completely cured even through a local enterectomy. In the absence of direct evidence of acquired von Willebrand syndrome (AVWS) or angiodysplasia, her long-standing gastrointestinal bleeding was finally stopped after receiving transcatheter aortic valve implantation (TAVI).
    METHODS: A 64-year-old female suffered from refractory gastrointestinal bleeding and exertional dyspnoea. A local enterectomy was performed owing to persistent hemorrhage and repeated transfusions; subsequently, histological examination revealed angiodysplasia. Heyde\'s syndrome was not suspected until 3 years later, at which time the patient started bleeding again and was also found to have severe aortic valve stenosis upon echocardiography. TAVI was consequently performed when the patient was in a relatively stable condition even though the predisposition to bleed, but there was no evidence of angiodysplasia and AVWS during angiography at that time. The patient\'s above symptoms were significantly relieved after TAVI and followed up for 2 years without any significant ischemic or bleeding events.
    CONCLUSIONS: The visible characteristics of angiodysplasia or a shortage of HMWM-vWFs should not be indispensable for the clinical diagnosis of Heyde\'s syndrome. Enterectomy could be a bridging therapy for aortic valve replacement in patients with severe hemorrhage, and TAVI may be beneficial for moderate to high surgical-risk patients even if they have a potential risk of bleeding.
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  • 文章类型: Journal Article
    目的:结肠血管发育不良是一种罕见的疾病,然而,它是老年人下胃肠道(GI)出血的常见原因。该研究总结了结肠血管发育不良的结肠镜和临床特征,以提高内镜医师对该疾病的认识。
    方法:我们对2013年9月至2022年4月间诊断为结肠血管发育不良的入选患者进行了回顾性研究。分析活动性出血患者的临床和结肠镜特征,并与未出血患者进行比较。还在结肠血管发育不良引起的活动性下消化道出血患者和其他疾病患者之间进行了比较。
    结果:总计,54名符合条件的患者被纳入本研究;55.55%的参与者年龄在60岁以上。10例结肠血管增生患者(3名男性和7名女性)患有活动性下消化道出血,主要位于左侧和全结肠。2型糖尿病患者,放疗史,抗血小板药物的使用,多发性病变更有可能忍受下消化道出血。结肠血管增生组出血和入院时间比其他疾病组长(P=0.043)。在结肠血管发育不良组中,3例患者出血复发,复发率高于其他疾病组(P<0.001)。
    结论:内镜医师应谨慎进行结肠镜检查,并将结肠血管发育不良作为下消化道出血患者的鉴别诊断,尤其是老年妇女和患有慢性疾病的成年人,如2型糖尿病。
    OBJECTIVE: Colonic angiodysplasia is a rare disease, it is nevertheless a common cause of lower gastrointestinal (GI) bleeding in older adults. The study summarized the colonoscopic and clinical features of colonic angiodysplasia to raise awareness among endoscopists regarding this disease.
    METHODS: We performed a retrospective study of enrolled patients diagnosed with colonic angiodysplasia between September 2013 and April 2022. Clinical and colonoscopic features of the patients with active bleeding were analyzed and compared with those of patients without bleeding. The comparisons were also conducted between the patients with active lower GI bleeding caused by colonic angiodysplasia and those by other diseases.
    RESULTS: In total, 54 eligible patients were included in this study; 55.55% of the participants were aged over 60 years. Ten patients (3 men and 7 women) with colonic angiodysplasia suffered from active lower GI bleeding, which was mainly located in the left and total colon. The patients with type 2 diabetes mellitus, radiotherapy history, antiplatelet drug use, and multiple lesions were more likely to endure lower GI bleeding. The duration between bleeding and admission was longer in the colonic angiodysplasia group than in the other diseases group ( P = 0.043). In the colonic angiodysplasia group, bleeding relapsed in 3 patients, and the recurrence rate was higher than in the other diseases group ( P < 0.001).
    CONCLUSIONS: Endoscopists should perform colonoscopy scrupulously and consider colonic angiodysplasia as a differential diagnosis in patients with lower GI bleeding, especially for older women and adults with chronic diseases, such as type 2 diabetes mellitus.
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  • 文章类型: Journal Article
    背景:小肠血管畸形(血管增生)是小肠出血的常见原因。虽然胶囊内窥镜检查已成为血管发育不良的主要诊断方法,手工阅读整个胃肠道既耗时又需要繁重的工作量,影响诊断的准确性。
    目的:评估人工智能是否可以辅助诊断并提高小肠血管增生的检出率,实现疾病自动检测,缩短胶囊内镜(CE)读取时间。
    方法:具有特征融合方法的卷积神经网络语义分割模型,在CE下自动识别血管发育不良的类别并绘制病变轮廓,从而提高了小肠血管畸形病变的识别效率和准确性,被提议了。以Resnet-50为骨架网络设计融合机制,融合浅层和深度特征,并对图像进行像素级分类,实现血管发育不良的分割和识别。构建训练集和测试集,并与PSPNet进行比较,Deeplab3+,和UperNet。
    结果:研究中构建的测试集取得了令人满意的结果,像素精度为99%,平均交叉超过联合为0.69,阴性预测值为98.74%,阳性预测值为94.27%。模型参数为46.38M,浮动计算为467.2G,分割和识别图像的时间长度为0.6s。
    结论:基于深度学习构建分割网络来分割和识别血管增生病变是诊断血管增生病变的有效可行方法。
    BACKGROUND: Small intestinal vascular malformations (angiodysplasias) are common causes of small intestinal bleeding. While capsule endoscopy has become the primary diagnostic method for angiodysplasia, manual reading of the entire gastrointestinal tract is time-consuming and requires a heavy workload, which affects the accuracy of diagnosis.
    OBJECTIVE: To evaluate whether artificial intelligence can assist the diagnosis and increase the detection rate of angiodysplasias in the small intestine, achieve automatic disease detection, and shorten the capsule endoscopy (CE) reading time.
    METHODS: A convolutional neural network semantic segmentation model with a feature fusion method, which automatically recognizes the category of vascular dysplasia under CE and draws the lesion contour, thus improving the efficiency and accuracy of identifying small intestinal vascular malformation lesions, was proposed. Resnet-50 was used as the skeleton network to design the fusion mechanism, fuse the shallow and depth features, and classify the images at the pixel level to achieve the segmentation and recognition of vascular dysplasia. The training set and test set were constructed and compared with PSPNet, Deeplab3+, and UperNet.
    RESULTS: The test set constructed in the study achieved satisfactory results, where pixel accuracy was 99%, mean intersection over union was 0.69, negative predictive value was 98.74%, and positive predictive value was 94.27%. The model parameter was 46.38 M, the float calculation was 467.2 G, and the time length to segment and recognize a picture was 0.6 s.
    CONCLUSIONS: Constructing a segmentation network based on deep learning to segment and recognize angiodysplasias lesions is an effective and feasible method for diagnosing angiodysplasias lesions.
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  • 文章类型: Journal Article
    Objective To explore the characteristics and clinical outcomes of patients with Heyde syndrome (HS) who undergo aortic valve replacement (AVR). Methods Electronic databases including PubMed, Embase, Ovid, WANFANG, VIP and CNKI were searched to identify all case reports of HS patients undergoing AVR surgery, using different combinations of search terms \"Heyde syndrome\", \"gastrointestinal bleeding\", \"aortic stenosis\", and \"surgery\". Three authors independently extracted the clinical data including the patients\' characteristics, aortic stenosis severity, gastrointestinal bleeding sites, surgical treatments and prognosis. Results Finally, 46 case reports with 55 patients aging from 46 to 87 years, were determined eligible and included. Of them, 1 patient had mild aortic stenosis, 1 had moderate aortic stenosis, 42 had severe aortic stenosis, and 11 were not mentioned. Gastrointestinal bleeding was detected in colon (n=8), jejunum (n=6), ileum (n=4), cecum (n=3), duodenal (n=3) and multiple sites (n=8). No specific bleeding site was identified in 23 patients. Preoperative hemoglobin level ranged from 43 to 117 g/L. All but one of 16 patients showed decreased level of high molecule weight von Willebrand factor. Of the 55 patients, 43 underwent AVR, and 12 received transcatheter AVR. Aortic valves of 14 cases were replaced by mechanical valves, and 33 cases by biological valves. All patients recovered well during the follow-up, except 5 patients. One patient who had perivalvular leakage and gastrointestinal bleeding after AVR underwent the second AVR. Two patients had recurrent gastrointestinal bleeding. Two patients died of life-threatening acute subdural hematoma and multiple organ failure, respectively. Conclusions HS is a rare syndrome characterized by aortic stenosis and gastrointestinal bleeding. AVR is an effective treatment for HS.
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